Inflammatory demyelinating chronic polyneuropathy: a contribution to the characterization of the disease
Correspondencia: Dra. Alicia V. Cabrera-Lima. Centro Internacional de
Restauración Neurológica, CIREN. Ave. 25 # 15805 e/ 158 y 160, Playa. CP 11300 La Habana, Cuba. Fax: (537) 336339, 336028, 336302.
E-mail: acabrera@neuro.sld.cu
Introduction and objective: The chronic inflammatory demyelinating polyneuropathy (CIDP) is a controversial sickness. No doubt it is still a diagnosis without a strict nosologic delimitation until we acquire a greater knowledge about the underlying mechanisms, or whether a specific marker is found. We evaluated its clinical and laboratory findings, to contribute to its characterization in our environment.
Patients and methods: We reviewed the records of 37 patients diagnosed with CIDP between 1986 and 1997. They were grouped in as to sex, age, beginning age (BA), evolution time, race, clinical form, symptoms, signs and evolutive profile. We analyzed the cytochemic and immunologic studies of cerebrospinal fluid (CSF) as well as sural nerve biopsy.
Results and conclusions: We demonstrated a predominance of BA between the fifth and sixth decades, and the presentation of a mixed polyradiculoneuropathy with motor predominance and a chronic progressive evolution. It was positively correlated with protein concentration levels in CSF, elevated in the 73.5% of the patients. There was an increase in the permeability of the blood brain barrier (BBB) in 50.8%, IgG intrathecal synthesis in 5.08 and oligoclonal bands in 8.8%. We found lost of myelin sheats in 90.6% of sural nerves and onion-bulbs formation in 60.5%, which demonstrated the high sensitivity of this study to confirm the diagnosis of demyelinating process as essential substrate in the illness.
Pacientes y métodos Revisamos las historias clínicas de 37 pacientes con este diagnóstico entre 1986 y 1997. Todos cumplían los criterios diagnósticos de Dyck et al (1992) y fueron evaluados mediante la escala para la neuropatía periférica Neurological Symptoms Score (NSS) y Neurological Disability Score (NDS), agrupados según sexo, edad, edad de comienzo, tiempo de evolución, raza, forma clínica, síntomas y signos, y perfil evolutivo. Se analizaron los resultados de los estudios citoquímico e inmunológico del LCR y de la biopsia de nervio sural. Resultados y conclusiones. Se demostró un predominio de la edad de comienzo entre la quinta y sexta década y de la presentación en forma polirradiculoneuropática mixta con predominio motor, con evolución crónica progresiva, correlacionada positivamente con la proteinorraquia, elevada en el 73,5%. Hubo incremento de la permeabilidad de la BHE (50,8%), síntesis intratecal de IgG (5,08%) y bandas oligoclonales (8,8%). Se constató pérdida de vainas de mielina en el 90,6% y formación de bulbos de cebolla en el 60,5% de las muestras de nervio sural, demostrándose la elevada sensibilidad de este estudio para la confirmación diagnóstica del proceso desmielinizante como sustrato esencial en la enfermedad